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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1989

Lymphokine-activated killer cytotoxicity in neonatal mononuclear cells: in vitro responses to tumor cell lines from pediatric solid tumors.

The presence of neonatal (cord) lymphokine-activated killer (LAK) cell activity toward natural killer cell resistant Raji and Daudi cell lines has recently been reported from our laboratory. We investigated the future therapeutic use of LAK adoptive immunotherapy by examining LAK in vitro cytotoxicity from both neonatal and adult mononuclear cells against solid tumor cell lines of relevance to pediatric oncology: SH-SY5Y (neuroblastoma), SK-NM-C (neuroblastoma-neuroepithelioma), NEP-1 (Wilms' tumor), SK-ES-1 (Ewing's sarcoma), and A-204 (rhabdomyosarcoma). Cord and adult mononuclear cells were activated by recombinant IL-2 (100 mu/ml) for 5-7 days and added in an effector:target ratio of 40:1 to 51Cr-labeled target cells. Specific cell lysis was determined after a 4-h incubation. There was a significantly high level of cord and adult LAK cytotoxicity against Wilms' (76.4 +/- 9.8 versus 77.3 +/- 6.8%) and Ewing's (84.2 +/- 5.5 versus 71.1 +/- 6.5%) cell lines and significant but moderate LAK activity against neuroepithelioma (52.0 +/- 6.6 versus 55.4 +/- 4.5%) and rhabdomyosarcoma (46.6 +/- 5.7 versus 43.9 +/- 5.2%) cell lines. There was no difference between cord and adult LAK activity toward these targets. However, a differential response toward the more classical neuroblastoma cell line, SH-SY5Y, was noted with significantly more LAK cytotoxicity from cord mononuclear cells than adult mononuclear cells (51.2 +/- 6.9 versus 28.5 +/- 8.2%) (p less than or equal to 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Antibody-Dependent Cell Cytotoxicity↗
PubMed1999

High expression of somatostatin receptor subtype 2 (sst2) in medulloblastoma: implications for diagnosis and therapy.

Medulloblastoma is a pediatric malignancy, which arises in cerebellum. The neuropeptide somatostatin (SS-14) is a neuromodulator and growth regulator in the developing cerebellum. SS-14 has previously been demonstrated in medulloblastomas with immunohistochemical techniques, but somatostatin receptor (sst) expression is less well understood. We analyzed somatostatin and sst subtype expression (sst1-5) in central primitive neuroectodermal tumors (cPNET), including 23 medulloblastomas, 6 supratentorial PNET, and 10 cPNET cell lines. The expression of SS-14 and sst genes in cPNET was compared with expression of these genes in 17 tumors of the Ewing's sarcoma family of tumors using reverse transcriptase-PCR, Southern hybridization, quantitative in vitro receptor autoradiography, and competitive membrane binding assays. The sst1 subtype was expressed in similar frequency in cPNET (83%) and Ewing's sarcoma family of tumors (71%). Nine of the 10 cell lines and 76% of the cPNET expressed mRNA for sst2 compared with 35% of the Ewing's sarcoma family of tumors. High-affinity binding of SS-14 was demonstrated in cPNET by quantitative autoradiography as well as by competitive binding assays. The cPNET cell line D283 Med bound SS-14 and octreotide with high affinity; SS-14 inhibited proliferation of D283 Med cells as measured by a decrease in [3H]thymidine uptake. We conclude that both sst1 and sst2 are highly expressed in cPNET and suggest that somatostatin may regulate proliferation and differentiation in these developmental tumors.

Adolescent↗
PubMed1989

Expression of the cholecystokinin gene by cultured human primitive neuroepithelioma cell lines.

In this report we identify two cultured human primitive neuroepithelioma cell lines that express cholecystokinin (CCK) RNA and synthesize substantial amounts of pro-CCK, but differ in their ability to process the prohormone. Seven cultured human neural tumor lines, including two primitive neuroepitheliomas and five neuroblastomas, were screened for CCK gene expression using a CCK C-terminal RIA system, a RIA specific for the carboxy-terminal extension of prepro-CCK, and RNAse protection assays specific for CCK mRNA. RNA derived from the two primitive neuroepitheliomas yielded strong hybridization signals with CCK-specific probes. The five other neuronal tumors were negative for CCK mRNA. The two primitive neuroepitheliomas also synthesized substantial quantities of material reactive in the CCK carboxy-terminal extension RIA system. One of the tumors, Sk-N-Mc, postranslationally processed the CCK prohormonal material poorly, yielding only high mol wt CCK precursors and no immunoreactive CCK. The other, SK-PN-Dw, was able to process the prohormone, producing immunoreactive CCK-like material plus a peptide that immunochemically and chromatographically resembled the intact CCK C-terminal extension peptide. These cultured tumor lines should prove useful in furthering our understanding of the regulation of the CCK gene in human neuronal cells and will also provide an in vitro system for investigation of the posttranslational processing of the CCK preprohormone. In addition, the data demonstrate that screening procedures for examining peptide hormone expression by tumors are most comprehensive when specific molecular genetic probes are employed in addition to standard peptide assay methodology. Finally, these data suggest that CCK production may be a feature of some primitive neuroepitheliomas.

Animals↗
PubMed1994

Turcot's syndrome: evidence for linkage to the adenomatous polyposis coli (APC) locus.

OBJECTIVE: To investigate the possibility that neuroepithelial tumors in Turcot's syndrome are caused by pleiotropic mutations in the gene for adenomatous polyposis coli (APC), a tumor-suppressor gene implicated in colonic cancer. METHODS: We studied the inheritance patterns of genetic markers for the chromosome 5q21 region in 12 members of a Turcot's syndrome kindred with five affected members. We performed linkage analysis to detect linkage between the disease phenotype and DNA markers. RESULTS: Marker D5S346, located 30 to 70 kilobases from the APC locus, showed evidence highly suggestive of linkage to the disease phenotype (lod score = 1.92). CONCLUSIONS: The data provide evidence that the tumor-suppressor gene implicated in APC and sporadic colon cancers may also cause malignant neuroepithelial tumors in Turcot's syndrome.

Adenomatous Polyposis Coli↗
PubMed1986

Late intracranial metastasis from esthesioneuroblastoma: case report and review of the literature.

Review of the literature reveals much disparity regarding the incidence, natural history, and survival rate in patients harboring esthesioneuroblastoma (olfactory neuroblastoma). The metastatic tendencies of this tumor have been highly variable in the literature, and reported intracranial involvement has been via local extension from the tumor's origin near the cribriform plate. Our present case describes a metastasis of esthesioneuroblastoma to the right parietal cortex and meninges in a patient without evidence of local recurrence after combined surgical and radiation therapy 5 years previously. The broad spectrum of biological behavior of this tumor, and its response to various therapies are discussed. The immunohistochemical and ultrastructural features of this unusual tumor are also presented. Recommendations are made for the management of these patients, including regular serial neurological examinations, serial computed axial tomography or magnetic resonance imaging of the head and neck, and, in the initial management, chemotherapy.

Adult↗
PubMed1988

Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases.

This report concerns the clinicopathological features of 39 cases of a morphologically unique and surgically curable group of neuroepithelial tumors associated with medically intractable partial complex seizures. All were supratentorial and characterized by intracortical location, multinodular architecture, and heterogeneity in cellular composition. The constituent cells included astrocytes, oligodendrocytes, and neurons. Because neuronal atypia was often inapparent, the tumors superficially resembled mixed oligoastrocytomas. The term "dysembryoplastic neuroepithelial tumor" (DNT) is proposed for these distinctive lesions, the clinicopathological features of which suggest a dysembryoplastic origin. With the exception of the occurrence of headaches in 2 patients, partial complex seizures were the exclusive symptom. Age at onset of symptoms ranged from 1 to 19 years (mean 9 years). In addition to the chronic nature of the seizures (range, 2 to 18 years; mean, 9 years), one-third of the patients showed radiological features, such as focal cranial deformity, indicating that the tumors had an early onset and were of long standing. In most cases, computed tomography showed a "pseudocystic," well-demarcated, low density appearance associated in some cases with focal contrast enhancement (18%) or calcific hyperdensity (23%). The tumor involved the temporal lobe in 24 patients (62%), the frontal lobe in 12 (31%), and the parietal and/or occipital lobe in 3 cases. Although tumor removal was considered incomplete or subtotal in 17 patients (44%), long term follow-up (range, 1 to 18 years; mean, 9 years) showed neither clinical nor radiological evidence of recurrence in any patient. Comparison of the survival data of the 13 subjects who had undergone postoperative radiotherapy with 26 who had not indicated that radiation therapy was of no obvious benefit. The identification of DNT has therapeutic and prognostic implications because aggressive therapy can be avoided, thus sparing these young patients the deleterious long term effects of radio- or chemotherapy.

Adolescent↗
PubMed1989

Surgical treatment of tumors in the anterior skull base using the transbasal approach.

Five patients with tumors in the anterior skull base were surgically treated using the transbasal approach, which permits removal of the tumor, repair of the dura mater, and reconstruction of the skull base in a one-stage procedure. By using autologous materials for the bone graft and pedunculated pericranial flap for the reconstruction, the intracranial structures are separated from the air-filled nasal and paranasal cavities. No postoperative complications such as wound infection and leakage of cerebrospinal fluid were encountered. The use of this surgical technique makes it possible to extirpate brain tumors that heretofore have been considered unresectable.

Adenoma↗
PubMed1992

Late manifestations of esthesioneuroblastomas in the central nervous system: report of two cases.

Two patients with esthesioneuroblastomas that disseminated in the central nervous system (CNS) and elsewhere are reported. The latest recurrences in the CNS were 19 years after the diagnosis in patient and 9 years after the diagnosis in the other. Because the treatment of patients with such late recurrences is complicated by previous surgical procedures and radiation therapy, we review therapeutic options for these patients. The literature has not previously stressed the occurrence of late metastases, including those to the CNS, after prolonged disease-free intervals.

Adolescent↗
PubMed1993

Esthesioneuroblastoma: prognosis and management.

Forty-nine patients with esthesioneuroblastoma were treated at the Mayo Clinic between 1951 and 1990. Their clinical manifestations and treatment results were reviewed to identify possible prognostic factors. The 5-year survival rate for all patients was 69%. Tumor progression occurred in 25 patients (51%; no local control in 6 and local recurrence in 19). Metastasis was found in 15 patients (31%; regional in 10 and distant in 9). Nineteen patients died directly from metastatic or intracranial tumor extension. The pathological grade of the tumor was the most significant prognostic factor identified. The 5-year survival rate was 80% for the low-grade tumors and 40% for the high-grade tumors (P = 0.0001). Surgical treatment alone is effective for low-grade tumors if tumor-free margins can be obtained. Radiation is used for low-grade tumors when margins are close, for residual or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Recurrent tumor and regional metastasis should be treated aggressively because this approach has been shown to be worthwhile. A craniofacial resection is now the surgical procedure performed in all cases. Because recurrence can occur after 5 or even 10 years, long-term follow-up is mandatory.

Adolescent↗
PubMed1995

Dysembryoplastic neuroepithelial tumor: morphological, immunocytochemical, and deoxyribonucleic acid analyses in a pediatric series.

Overtreatment by radiotherapy and/or chemotherapy for central nervous system tumors in infancy and childhood may be deleterious, so the recognition of surgically curable clinicopathological entities is mandatory. The dysembryoplastic neuroepithelial tumor is a complex multinodular lesion consisting of glial nodules, associated with a specific glioneuronal element and/or with focal cortical dysplasia, and occurring in young patients presenting with intractable, mostly complex partial, seizures without neurological deterioration. We report on 14 patients; 9 were from a series of 600 pediatric patients with intracranial central nervous system tumors studied at a single institution from 1988 to 1993, and 5 were referred from other pediatric hospitals. Six tumors were frontal, six were temporal, one was parietal, and one was occipitoparietal. Computed tomographic scans disclosed hypodense lesions with cystic appearances in 4 patients and slight focal postcontrast enhancements in only 2 patients, whereas magnetic resonance imaging, available for 7 of 14 patients, showed hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images. Deformities of the overlying cranium were also observed in five patients. The age range at the time of surgery (excluding a 20-year-old male patient who underwent surgery at the main pediatric hospital) was 2.6 to 13 years, with a mean of 6.68 years. The male to female patient ratio was 10:4, and the duration of symptoms was 0.2 to 6 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1979

Brain radiation necrosis following treatment of an esthesioneuroblastoma (olfactory neurocytoma).

Esthesioneuroblastoma is an uncommon malignant nasal tumor which may be difficult to diagnose. Once diagnosed, selection of treatment may be a dilemma. Some advocate preoperative radiation and surgery; others, surgery and postoperative radiation if needed. A case that developed brain necrosis from postoperative radiation is presented. It is felt that the possibility exists that the patient might still be living with tumor had irradiation not been given. This is a plea for the conservative approach to treatment. The author agrees with John S. Lewis that it is unwise to use all modalities at once as there is no definite evidence that any one method of treatment or a combination of surgery and irradiation will alter the prognosis. The paper stresses the brain hazards of irradiation.

Adult↗
PubMed1981

Pericranial flap for the closure of defects of craniofacial resection.

Malignant neoplasms involving the ethmoid sinuses mandate en bloc resection via the craniofacial approach. This technique leaves a direct communication between the nasal chamber and the extradural space to be closed. The elevation of dura from the nasal chamber and the extradural space to be closed. The elevation of dura from the anterior chamber and the extradural space to be closed. The elevation of dura from the anterior cranial fossa and cribriform area may result in small dural tears, CSF leak and/or CNS infections. We have utilized an inferiorly based pericranial flap and a laterally based osteoplastic bone flap to successfully close this space and dural defects. The laterally based bone flap allows insetting pericranial flap without compromising the vascularized inferior base and gives generous exposure of the area to be resected. This approach has been used successfully in four patients in the last year. Three patients had esthesioneuroblastomas and the fourth had a squamous cell carcinoma of the maxilloethmoid complex. This communication describes the technique and lists advantages of the inferiorly based pericranial flap.

Adolescent↗
PubMed1984

Olfactory neuroblastoma: presentation of a case and review of the Japanese literature.

Olfactory neuroblastoma is an uncommon malignant tumor arising from the nasal cavity. During the past 18 years from 1964 to 1982, only 24 cases have been in Japanese literature, including this case. The average age of these patients is 42.3 years, ranging from 4 to 75 years old. The sex ratio of male to female is 10:14, female being prevalent moderately. The peak distribution is from 30 to 40 years old. The majority of the patients died of distant metastasis within 2 years of diagnosis. Therefore, the overall prognosis in Japan is very poor, as compared with about a 50% 5 year survival rate in the western countries. This is probably because this tumor is difficult to diagnose correctly, and the chance to start treatment early is apt to be missed here, though some tumors behaved very aggressively from the beginning, metastasizing early.

Adolescent↗